PT - JOURNAL ARTICLE AU - William K Diprose AU - Anthony Jordan AU - Neil E Anderson TI - Autoinflammatory syndromes in neurology: when our first line of defence misbehaves AID - 10.1136/practneurol-2021-003031 DP - 2022 Apr 01 TA - Practical Neurology PG - 145--153 VI - 22 IP - 2 4099 - http://pn.bmj.com/content/22/2/145.short 4100 - http://pn.bmj.com/content/22/2/145.full SO - Pract Neurol2022 Apr 01; 22 AB - Autoinflammatory syndromes result from a defective innate immune system. They are characterised by unexplained fever and systemic inflammation involving the skin, muscle, joints, serosa and eyes, along with elevated acute phase reactants. Autoinflammatory syndromes are increasingly recognised as a cause of neurological disease with a diverse range of manifestations. Corticosteroids, colchicine and targeted therapies are effective if started early, and hence the importance of recognising these syndromes. Here, we review the neurological features of specific autoinflammatory syndromes and our approach (as adult neurologists) to their diagnosis.